Symptomatic management

Patients with MPS VII can have respiratory and cardiovascular complications, skeletal manifestations, loss of hearing and vision, and gastrointestinal symptoms.3

Symptomatic management may include:1,3

Oxygen for respiratory insufficiency

Continuous positive airway pressure (CPAP) machines

Tracheotomy for severe airway obstruction

Physical therapy for joint stiffness

Cardiac valve replacement therapy

Anesthesia, surgical interventions, and emergencies

Patients with MPS VII may have cervical cord compression, cervical instability, and compressed airways. Giving an anesthetic to such patients requires skill and should always be undertaken by an experienced anesthetist.1-3

If the patient must go to a different hospital in the event of an emergency, be sure to inform the anesthetist that there may be problems with intubation. Also work with the MPS VII patient and their family to help inform their school, work, and any other caregivers of these concerns.1-3

Physical therapy/exercise3

Joint stiffness and limitation of motion are common features of MPS VII and can cause significant loss of function.

Range-of-motion exercises (passive stretching and bending of the limbs) may offer some benefits in preserving joint function, and should be started early.